Review current literature and guidelines for malignant hyperthermia in the context of neurotologic surgery.A case of malignant hyperthermia during vestibular schwannoma surgery, in a patient previously exposed to anesthesia.Excision of vestibular schwannoma, acute management of malignant hyperthermia.Knowledge of the basic pathophysiology, clinical manifestations, and treatment protocols for malignant hyperthermia.Rapid termination of the procedure and appropriate modifications in surgical technique permitted expeditious treatment of malignant hyperthermia and prevented its lethality.Malignant hyperthermia is a rare and lethal condition that may arise in neurotologic surgery, even in patients who have previously received general anesthesia. The neurotologic surgeon has a role in early recognition and expeditious termination of surgery to help reduce its mortality.